(989) Intrathecal Morphine Delivered by Baclofen Pump for Status Dystonicus in Lesch-Nyhan Syndrome

Introduction: Lesch-Nyhan syndrome is an X-linked recessive disorder caused by a deficiency of hypoxanthine-guanine phosphoribosyltranserase. Clinical features include dystonia, nephrolithiasis, impaired renal function, acute gouty arthritis, and self-mutilating behaviors. The disease is extremely debilitating and is often fatal by the third or fourth decade of life. Limited literature exists supporting intrathecal opiates in treatment of status dystonicus.

Description: A 15-year-old male with Lesch-Nyhan syndrome was admitted with one month of progressive dystonia. At presentation, the patient had rhabdomyolysis, and was opisthotonic and non-communicative due to severe dystonia. After excluding common causes of dystonia, the dystonia was thought to be caused by visceral hyperalgesia. Throughout the 42 hospitalization days (HD), he received intermittent benzodiazepines (HD 2-33 ), ketorolac (HD 1-23; 22 total doses), both intermittent (HD 3-24) and continuous infusions (HD 24-32) of ketamine, dantrolene (HD 3-41), prucalopride (HD 7-41), hydromorphone (HD 7-40), methadone (HD 15-20), cyclobenzaprine (HD 7-37), hyoscyamine (HD 13-39), amitriptyline (HD 19-41), and dexmedetomidine infusion (HD 20-35). Intrathecal baclofen was increased by 10% on HD 7 and HD 14. These medications did not provide relief of symptoms. Spinal anesthesia was considered but not performed due to the severity of scoliosis and opisthotonos. Gabapentin, previously avoided for history of allergic reaction, was started on HD 28. On HD 29, morphine was added to the intrathecal baclofen pump at a dose of 0.1 mg/day. Rapid improvement in symptoms was observed on HD 32. Medications were subsequently weaned and patient was discharged on HD 42.

Discussion: While it is difficult to determine whether morphine or gabapentin resulted in symptom relief, rapid improvement after initiation of these medications must be appreciated. Use of patients’ preexisting intrathecal baclofen pump to simultaneously deliver opiates is not well described in the literature. When the spine is inaccessible, utilization of preexisting intrathecal baclofen pump should be considered for delivery of opiates. More research is needed regarding long term safety, efficacy, and timing to optimize therapy and avoid polypharmacy.